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Published in: Clinical Rheumatology 4/2021

01-04-2021 | Aneurysm | Original Article

Non-coronary cardiac events, younger age, and IVIG unresponsiveness increase the risk for coronary aneurysms in Italian children with Kawasaki disease

Authors: Marianna Fabi, Laura Andreozzi, Ilaria Frabboni, Ada Dormi, Elena Corinaldesi, Francesca Lami, Cristina Cicero, Bertrand Tchana, Rosa Francavilla, Monica Sprocati, Barbara Bigucci, Claudia Balsamo, Paola Sogno Valin, Giorgia Di Fazzio, Lorenzo Iughetti, Enrico Valletta, Federico Marchetti, Andrea Donti, Marcello Lanari

Published in: Clinical Rheumatology | Issue 4/2021

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Abstract

Objectives

Kawasaki disease (KD) is the most frequent cause of acquired heart disease in children in high-income countries because of coronary artery involvement. Risk factors for coronary lesions can vary in consideration of different genetic background and environmental factors.

Methods

Multicenter retrospective and prospective study including 372 consecutive children (58% boys; mean age 34.3 ± 30.3 months, Caucasian 85%) was diagnosed with KD. We divided the cohort into 2 groups according to the presence of coronary anomalies (CAA) and aneurysms. We compared the groups and studied the risk factors for CAA and for aneurysms, the most severe lesions.

Results

Children with CAA were 91/372 (24.46%, aneurysms 20/372, 5.37%). Children with CAA were more likely to have a longer duration of fever (p < 0.001), later day of treatment (p < 0.001), to be IVIG non-responders and late treated (p < 0.001), while age, clinical presentation, and seasonality were not different. They also had significantly higher WBC and neutrophils, lower lymphocytes, Hb and Na during the acute stage, and slower resolution of inflammation. Age, IVIG unresponsiveness, and presence of non-coronary cardiac findings were independent risk factors for CAA and for aneurysms, while neutrophils just for CAA. Age under 6 months was a risk factor for the aneurysm. Aneurysms occurred more frequently in the first quartile of the age of KD onset (under 14 months).

Conclusion

Very young children with non-coronary cardiac findings are at increased risk for a more severe form of KD with aneurysms. These children could benefit from adjunctive therapy beside IVIG, especially if they have higher markers of inflammation, particularly neutrophils.
Key points
Risk factors for coronary lesions can vary in consideration of different genetic background and environmental factors.
Risk factors for coronary involvement have been extensively studied in the Asian population, and others have been validated in cohorts with mixed ethnicities.
In our predominantly Caucasian population, non-coronary cardiac findings, age younger than 6 months, and IVIG unresponsiveness are independent risk factors for a more severe form of KD with aneurysms.
Literature
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go back to reference Hamada H, Suzuki H, Onouchi Y, Ebata R, Terai M, Fuse S et al (2019) Efficacy of primary treatment with immunoglobulin plus ciclosporin for prevention of coronary artery abnormalities in patients with Kawasaki disease predicted to be at increased risk of non-response to intravenous immunoglobulin (KAICA): a randomised controlled, open-label, blinded-endpoints, phase 3 trial. Lancet 393:1128–1137. https://doi.org/10.1016/s0140-6736(18)32003-8CrossRefPubMed Hamada H, Suzuki H, Onouchi Y, Ebata R, Terai M, Fuse S et al (2019) Efficacy of primary treatment with immunoglobulin plus ciclosporin for prevention of coronary artery abnormalities in patients with Kawasaki disease predicted to be at increased risk of non-response to intravenous immunoglobulin (KAICA): a randomised controlled, open-label, blinded-endpoints, phase 3 trial. Lancet 393:1128–1137. https://​doi.​org/​10.​1016/​s0140-6736(18)32003-8CrossRefPubMed
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go back to reference Newburger JW, Takahashi M, Gerber MA, Gewitz MH, Tani LY, Burns JC et al (2004) Diagnosis, treatment, and long-term Management of Kawasaki Disease: a statement for health professionals from the committee on rheumatic fever, endocarditis, and Kawasaki disease, council on cardiovascular disease in the young, American Heart Association. Pediatrics 114:1708–1733. https://doi.org/10.1542/peds.2004-2182CrossRef Newburger JW, Takahashi M, Gerber MA, Gewitz MH, Tani LY, Burns JC et al (2004) Diagnosis, treatment, and long-term Management of Kawasaki Disease: a statement for health professionals from the committee on rheumatic fever, endocarditis, and Kawasaki disease, council on cardiovascular disease in the young, American Heart Association. Pediatrics 114:1708–1733. https://​doi.​org/​10.​1542/​peds.​2004-2182CrossRef
Metadata
Title
Non-coronary cardiac events, younger age, and IVIG unresponsiveness increase the risk for coronary aneurysms in Italian children with Kawasaki disease
Authors
Marianna Fabi
Laura Andreozzi
Ilaria Frabboni
Ada Dormi
Elena Corinaldesi
Francesca Lami
Cristina Cicero
Bertrand Tchana
Rosa Francavilla
Monica Sprocati
Barbara Bigucci
Claudia Balsamo
Paola Sogno Valin
Giorgia Di Fazzio
Lorenzo Iughetti
Enrico Valletta
Federico Marchetti
Andrea Donti
Marcello Lanari
Publication date
01-04-2021
Publisher
Springer International Publishing
Published in
Clinical Rheumatology / Issue 4/2021
Print ISSN: 0770-3198
Electronic ISSN: 1434-9949
DOI
https://doi.org/10.1007/s10067-020-05331-w

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